Intracranial medulloepithelioma is extremely rare and highly malignant in central nervous system. It is a kind of fast-growing tumor and shows a propensity to spread widely throughout the central nervous system. It commonly occurs in infants and young children. We reported a rare case of 2 years old female child with a huge mass diagnosed as medulloepithelioma. Though radiological examination was characteristic for the neoplasm, it was not enough to make a definite diagnosis. Combining with histopathological examination was contributing to diagnose medulloepithelioma and differentiate it from other central nervous system tumors. We aimed at more understanding and knowledge of intracranial medulloepithelioma via reporting this case.